When you live with a disease like KTS, you feel like a fish in a fishbowl. (image by wharman, flickr)
Learn what Klippel Trenaunay syndrome (KTS) is and how to live with it, from a 21 year old who was diagnosed with this rare congenital malformation at birth.
Despite the multiple procedures and surgeries she’s had, Arianna is positive about her life and determined to help others cope with the symptoms and treatments of illnesses such as KTS.
“It’s okay to have days where you are upset about your illness – you don’t have to hold everything in,” says Arianna, who is a full-time college student at Merrimack College. “If you do, it will boil over.”
When I post Survivor’s Stories, I like to include a book at the beginning of every article so readers can get more information. But, I didn’t find anything related to Klippel Trenaunay Syndrome on Amazon. There are lots of medical textbooks about “human malformation” and syndromes of the skin, head and neck, but nothing on coping with the symptoms and treatments of KTS.
Maybe Arianna will be the first to write a book about Klippel Trenaunay syndrome.
Learning how your thought patterns affect your physical health can help you live in harmony with chronic illness.
Here’s how Arianna copes with the symptoms of Klippel Trenaunay syndrome…
What is Klippel-Trenaunay Syndrome (KTS)?
This summary of the causes, symptoms, and treatments of Klippel-Trenaunay is from the National Institute of Neurological Disorders and Stroke:
Klippel-Trenaunay syndrome is a rare congenital malformation involving blood and lymph vessels and abnormal growth of soft and bone tissue. Typical symptoms include hemangiomas (abnormal benign growths on the skin consisting of masses of blood vessels) and varicose veins. Fused toes or fingers, or extra toes or fingers, may be present. In some cases, internal bleeding may occur as a result of blood vessel malformations involving organs such as the stomach, rectum, vagina, liver, spleen, bladder, kidneys, lungs, or heart. Individuals are also at risk for blood clots.
The cause of Klippel-Trenaunay syndrome is unknown. A similar port-wine stain disorder in which individuals have vascular anomalies on the face as well as in the brain is Sturge-Weber syndrome. These individuals may experience seizures and mental deficiency. In some cases, features of the Klippel-Trenaunay syndrome and Sturge-Weber syndrome coincide. Another overlapping condition is the Parkes-Weber syndrome, which is characterized by abnormal connectivity between the arterial and venous system (arteriovenous fistulas).
There is no treatment or cure for KTS. Laser surgery can diminish or erase some skin lesions. Surgery may correct discrepancies in limb size, but orthopedic devices may be more appropriate. KTS is often a progressive disorder, and complications may be life-threatening. However, many individuals can live well while managing their symptoms.
How Arianna Copes With Klippel Trenaunay Syndrome
My Klippel Trenaunay has flared often over the years. Growing up, I had a multitude of procedures and hospitalizations but was still able to live somewhat of a normal life. However, when I hit high school (literally the first week of my freshman year), I started getting severe cellulitis infections on a constant basis.
For the next four years, I was shuffled in and out of the hospital along with having several operations. I also have dealt with blood clots my entire life.
Tips for Living With Klippel Trenaunay Syndrome
Surround yourself with people who are supportive and will listen to you. Keeping yourself surrounded by positive people is essential; as much as possible, try not to associate yourself with negative ones.
My particular illness impacts its patients in various ways, so it would be hard for me to give another KTS patient specific advice based upon the condition itself.
If your loved one has been diagnosed with Klippel Trenaunay syndrome, expect that they’ll need time to themselves some days to cope with everything they have going on. Don’t be offended if they request some time alone. If they decline an invitation to go out, don’t assume they are just bailing.
Chronic illness is exhausting physically and mentally, and not everyone realizes the toll it takes on one’s mind.
If your skin is severely affected by Klippel Trenaunay syndrome, you may find Psoriasis – How Alisha Copes With the Symptoms of Skin Disease helpful.
Does Arianna Live in Harmony With Her Illness – or Fight It?
Both. Klippel Trenaunay syndrome is very uncommon and not known by most, so I feel it is important to speak up for it as much as I can, to promote awareness and research. I along with my KTS colleagues have all significantly been impacted by this disease and it gets progressively worse as we age. I want to help ensure a better life for those who will be impacted by this condition in the future.
Arianna Faro
Klippel Trenaunay syndrome has motivated me to do an array of things. About four years ago, I wrote an article for Children’s Hospital Boston’s Dream magazine called A rare disfiguring disease won’t stop me. And, I did a small documentary: Arianna talking about Klippel-Trenaunay
This summer, I will be speaking at the national KTS conference in front of several doctors and patients. I was discovered through an old blog of mine in which no longer exists, but I have recently started a new one that will chronicle journeying through life with chronic illness- and the anxiety(OCD)/depression that ensued following all the medical chaos.
It is a constant battle, but I have learned so much over the years and hope to help others like me someday.
If you have any questions for Arianna, find her on twitter @ariannakts, email her at faroa@merrimack.edu, or visit her blog Toujours Belle (toujours belle means “still beautiful” in French. I chose the name because in spite of the abnormal difficulties we experience, we are still all beautiful beings both inside and out).
And if you have any thoughts about Klippel Trenaunay syndrome – or you want to share your experience with it – please comment below!
Hi Arianna,
Thank you so much for sharing that with us!
I am a 30 year old female from Melbourne, Australia. I have KTS in my left leg. I had two surgeries on my knee at ages 5 and 7, I have never let this affect my life and have led a relatively normal life, although I too get mild depression at times, only when the pain kicks in.
I have developed arthritis in my knee and ankle over the past 7 years and it has gradually worsened and has become very painful at times.
I will no doubt need to have a knee replacement sometime in the near future, but the doctor has advised that I deal with my current pain for as long as possible before attempting surgery.
Just wondering if anyone here has had a knee replacement or an ankle fusion with KTS? I am trying to find someone to speak to regarding these surgeries as I am scared of the possible complications that come with this surgery.
My email is suzana83@bigpond.com
Many thanks
Suzana
Hi,
My name is Amy and I have had a port wine stain that has covered my left arm, left chest and left shoulder since birth but it wasnt until I was in my late 30′s until I experienced the lymphedema and pain due to a car accident and was then diagnosed with KTS. It has been a struggle for me to wear the sleeve for it is uncomfortable. I have slowly come to realize that I must wear it as much as possible for it has gotten bigger in the last 2-3 yrs. I am now 46 yrs old and I am not going to let it beat me! I have gone back to school for a BSN at LSU school of nursing. I dont experience much pain anymore as long as I take my medication and wear the sleeve as much as possible, and most importantly I feel relief when I go to occupational therapy as an outpatient for lymphedema massage, but I do feel pain around the left shoulder blade muscle area and no one has an answer. Going to therapy really gives me relief for days. I take procardia XL which is a channel blocker to keep the vessels open and lyrica for peripheral polyneuropathy that comes and goes. I too enjoy when people stop me and ask me about my birthmark on my arm and I am able to educate them about it. The port wine stain has never bothered me only the swelling in it bothers me as for as looks go. I am proud to have had the help of Mrs. Erin Rosas to help diagnose me a few years back for I had went to numerous doctors and no one knew what I had. I was in a great deal of pain at that time due to flying when I didn’t know that I had this. Due to several flights my ulnar artery had basically constricted off and wasnt really functioning at all plus the build up of lymph fluid. I just recently went through lymphatic manual drainage last year with only slight reduction is swelling but a world of difference in an overall general health feeling and less pressure in the arm. I am currently searching for more answers to reduce the swelling in my arm and I am not going to give up with it!! If there is anyone that has any answers please let me know!
Dear Amy, I’m sorry nobody had any answers for you. It sounds like you’ve been through so much with your KTS and port wine stain! I’m glad you were diagnosed with Mrs Rosas’ help.
Have you found anything to help reduce the swelling in your arm?
Hi, my 6 year old daughter was diagnosed with KTS last year. She’s always had a port wine stain on her thigh, followed by venous malformations and varicose veins. Her middle toe on the leg is longer than the rest of her toes and there is huge vein leading up to it that is very thick and sticks out on her foot (It’s the same vein that is a varicose vein on the side of her calf muscle. She has a slight length difference between her legs, as well as width. She’s also got knock knees. Most people don’t notice it because it’s not as severe as some of the pictures I’ve seen online, but my concern is that I keep hearing that it progresses with time. For now, she is not at all affected by it, other than cosmetically. I see that you were diagnosed at 13, did you have any symptoms prior to your diagnosis? It seems like I have to do a lot of digging to find any info on KTS other than the regular websites that just describe what it is word for word as the other websites. Anyway, I stumbled across this page as I was trying to search all the back pages of the internet. Thanks for your blog!
Thank you for talking about KTS here. I don’t know much about it, and I admire people who aren’t just surviving it – they’re living fully and deeply!
When I first found out I had KTS I felt like my whole world came crashing in. At the time I knew I had a problem but having a name for it all scared me I was 13. And at first the doctors didn’t know what it was. They just knew how to fix it. Luckily I don’t have it as severe as most. But it is still a struggle. I started high school having to wear a supe tight compression stocking (always wearing pants because I hated people knowing I had this problem. I went through numerous surgeries to fix the veins because more kept forming and doctors even brought up amputation wich crushed me. But I pushed through and I’ve gone a year with out having a surgery on it because it has made mega improvements exercise helped it a lot I am now 17 and I am to the point where I don’t get pain all the time and to wear I don’t have to wear the compression stocking all the time. And as good as its doing I do still get frustrated but you know what I actually like showing of my birthmark (port wine stain) to everyone because I now love when they ask me about it and I tell them about my struggle with KTS and I’m proud to be able to say I dealing with it and I got through it d am living a for the most part normal life. Don’t ever give up no matter how hard it may seem.
You’re right Arianna and I think it’s fantastic what you are doing . I don’t know his many times doctors have said to me ”what, can you spelling that for me” when I’ve mentioned my condition.
So I’m sorry if you thought I was demeaning or minimizing anyone’s experience because I wasn’t. It is a spectrum condition and varies significantly from person to person, as do the symtoms. I think I’m probably frustrated with my KTS because I have no control over it yet it impacts me so significantly.
It was a frustrating, difficult, familiar day in an ongoing battle to gain control of my health.
Hi Lily! Thank you so much for your comment. I am so sorry to hear of all in which you must battle on a daily basis; I cannot even imagine how difficult that must be; dealing with just one condition is enough to exhaust me, so I give you a lot of credit as you must be an incredibly strong person/fighter!
With KTS, I think it is important to remember that it does manifest itself in each patient differently; sometimes on a dramatic scale, unfortunately. Some patients live a relatively normal life with it while others are born with it to a much more debilitating degree, overall impacting their quality of life significantly.
I also know of cases in which KTS impacts people’s internal organs. In fact, in one close KTS friend of mine who has the condition in his arm/chest, his malformation was trying to push his heart over and his family was told he would more than likely not survive because of this when he was a young baby (he is now in his twenties). So while I am lucky enough to not have my organs be impacted by KTS, there are unfortunately patients whose internal organs are greatly impacted by this condition.
Wishing you all the best!
Arianna
Thank you so much for your kind words, Mrs. Merlino! You have always been a positive force within my life and for that I am very much so grateful. Thank you for having been not only an excellent and inspirational teacher, but a true friend!
Much love,
Arianna
Laurie, my pleasure and thank you for helping to expose KTS to the world; I am so appreciative that you took the time to not only research the condition but to share that information with others.
A loyal fan of your blogs,
Arianna
Its all about perspective. Yeah Klippel Trenaunay Syndrome is a challenge and mine causes me severe pain and weakness in my affected leg but try KTS, Crohns disease & Graves disease. Out the three conditions I have my KTS is the least problematic of the three. The symptoms while not curable are manageable (to a certain degree) I can take active steps to looking after it and best of all I can see it – my other conditions affect my internal organs so I only get information on what is happening with them when I have hideous symptoms or the pain that feel like my body is going to crack apart.
I’ve always said I can cope with my KTS and it’s symptoms and it’s manifestations but having three conditions that all challenge your survival is a real roller coaster. When I wake up in the morning it’s a pot luck as to which
condition will rear its head that day and try to take me down.
So yes, KTS is the most unusual condition I have….but not the worst. It really is all about perspective.
I am a former teacher and later, friend, of Arianna’s. What most impresses me about her is her positive nature and her incredible spirit. She is one of the kindest, most caring people I know.
Her talent as a writer is evident and I think that your suggestion that she write a book about this disease is an excellent one.
She is truly a beautiful person, definitely from the inside/out.
Arianna, thank you for sharing about KTS with us! I’d never heard of it, and am glad that you’re speaking out about it. You sound like such a survivor – we need more people in the world like you!